Country singer joins Austin dad’s effort in finding cure for disease
By KVUE staff on KVUE.com
published January 13, 2012
An Austin father is on a desperate search to find a cure for a disease most people know very little about.
It’s called Batten Disease. It begins in childhood and is often fatal. Now his efforts have gotten the attention of country music superstar Keith Urban.
Co-founder of the Beyond Batten Disease Foundation Craig Benson spoke to KVUE about his daughter, who suffers from the disease.
To help out, you can attend a special event at the Austin City Limits Moody Theater. It’s called Date Night with Keith Urban. It’s taking place on Friday,Feb. 10 at 9 p.m.
Country Music Superstar Keith Urban Comes to Austin for Feb. 10 Gala Supporting Beyond Batten Disease Foundation
Austin, TX (PRWEB) November 02, 2011
Keith Urban – among country music’s biggest stars and a nominee for CMA’s 2011 Entertainer of the Year and Male Vocalist of the Year awards – will return to Austin for the first time in many years to perform at a Feb. 10 gala benefiting Beyond Batten Disease Foundation. The foundation fights Batten disease and also is working to eradicate hundreds of additional, serious and often fatal, inherited, childhood illnesses.
“Date Night with Keith Urban,” at ACL Live at The Moody Theater, will include a full concert performance by Urban, renowned for blockbuster shows, in Austin’s newest and arguably most spectacular music venue. The evening will begin with a “can’t miss” VIP pre-party attended by a who’s who list of guests from Austin, Dallas and Houston and an interactive auction featuring unique items and experiences.
The foundation is offering a variety of event sponsorship packages including some that provide a meet-and-photo opportunity with the superstar and accommodations at the hip W Hotel next to The Moody Theater (ideal for a memorable Valentine’s Day weekend getaway). Individual tickets, available exclusively through the Beyond Batten Disease Foundation website, go on sale to the public November 21.
Craig and Charlotte Benson of Austin started the foundation in 2008 after their then five-year-old daughter, Christiane, was diagnosed with Batten disease, a rare and fatal neurodegenerative disorder. Children born to parents who unknowingly each carry a gene mutation for Batten disease are at risk to inherit the condition which initially causes vision loss and seizures, then progressively impairs cognitive and motor capacities, and ultimately results in death during the late teen years or early 20s. Because Batten disease and so many other similar inherited, childhood diseases are uncommon, there is not much federal funding for research aimed at treatment and prevention.
In its three short years, the foundation has developed the most comprehensive carrier screening test panel for detecting genetic mutations that cause Batten disease and more than 600 other devastating, rare conditions that strike and kill thousands of kids each year. In addition to being so comprehensive, the test will be easy to administer and more economically priced than the cost of an individual test for any one of the diseases on the panel. The scientific and medical communities have heralded the test as a major breakthrough and it has received extensive media and professional journal coverage around the world.
Funds generated by “Date Night” will help the foundation to complete work to bring the test to market in 2012. The foundation plans to use proceeds from sales of the test to become self-sustaining for continuing Batten disease research.
“Keith Urban is one of the greatest entertainers of our day so we are ecstatic to have him join us at this exciting event,” said Shannon Janek, Event Co-Chair. “We expect his participation to boost awareness of Batten disease and the hundreds of other serious, inherited childhood diseases. He will be a huge draw, generating important financial support for the foundation and spotlighting its work, which is relevant for everyone planning to have children.”
According to foundation-funded research, on average, each person carries three genetic mutations that can cause a severe childhood disease or disorder. Carrier parents can even pass these mutations onto children who do not develop a condition. When these children mature and have kids, their children also are at risk of suffering from one of these conditions. Better-known examples of serious, inherited childhood diseases include Tay-Sachs disease, Cystic Fibrosis and Spinal Muscular Atrophy, or SMA. (One in 40 people are carriers for SMA.) A carrier screening strategy similar to the one that the foundation is developing has been used for Tay-Sachs disease and achieved an impressive 90 percent reduction in the incidence among at-risk populations.
About Keith Urban
Among today’s most celebrated country music artists, Keith Urban has been honored with Grammies and Australia’s coveted Aria Award as well as awards from the Country Music Association and the Academy of Country Music. New Zealand-born and Australia-raised, Urban moved to Nashville in 1992. His first American album came as a member of The Ranch (1997), followed by an increasingly accomplished series of multi Platinum-selling solo albums: Keith Urban (1999), Golden Road (2002), Be Here (2004), and Love, Pain & The Whole Crazy Thing (2006). The compilation Greatest Hits: 19 Kids (2008) included such No. One hits as “But For The Grace Of God,” “Somebody Like You” (named the top country song of the decade), “Who Wouldn’t Wanna Be Me,” “You’ll Think Of Me,” “Days Go By,” “Making Memories Of Us,” “Better Life,” and “You Look Good In My Shirt.” In his sixth and most recent album, Get Closer, Urban dives deeply into the exploration of love and relationships. For more information, please visit http://www.keithurban.net.
About Beyond Batten Disease Foundation
Beyond Batten Disease Foundation works to cure and prevent Batten disease, a rare, inherited neurological disorder that strikes young children, first causing vision loss and seizures, then cognitive and motor impairment, and ultimately death during the late teen years or early 20s. The foundation raises funds for research and is leading development of an easy and inexpensive, groundbreaking blood test to detect the gene mutations that cause Batten disease as well as 600-plus other rare but serious and often fatal childhood ailments. For more information, visit https://beyondbatten.org.
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Designing a Plan for Drug Discovery in Rare Pediatric Neurodegnerative Disease
by Danielle Kerkovich, Ph.D. and Amy Drew
published July 21, 2011
Editor’s Note: There are currently no cures for neurodegenerative diseases, including Batten disease, a rare and fatal disorder affecting young children. While researchers have made headway in preventing genetic disorders through preconception carrier screenings and have found potential drug targets, the gap between basic research and clinical treatment development remains. To overcome this gap, write authors Dr. Danielle Kerkovich and Amy Drew, researchers in academia and the pharmaceutical industry, supported by government agencies and nonprofit institutions, must come together to share expertise and promote translational research.
To read the full article, click here.
Father Fighting to End Batten Disease
Craig Benson and the Beyond Batten Disease Foundation were recently featured on My Fox Austin. Click here to view the segment.
published on July 1, 2011
Run to the Sun Relay benefits children with Batten Disease
By KVUE staff on KVUE.com
Published May 10, 2011
Batten Disease is a rare and fatal neurological disease that begins in childhood.
Craig Benson is a co-founder of the Beyond Batten Disease Foundation and Lance Thompson is a chairman of Run to the Sun Relay, which raises money for research in to Batten Disease. They spoke to KVUE.
Family starts foundation, raises money to fight Batten disease
by Andrew Ball in the Austin American Statesman
published May 8, 2011
Craig and Charlotte Benson had never heard of Batten’s disease before their then-5-year-old daughter was diagnosed with it in 2008.
But when the Austin couple learned Christiane was afflicted with the fatal illness, they threw themselves into finding a cure. In today’s column, Craig Benson writes about the nonprofit group he and his family started: the Beyond Batten Disease Foundation.
What is the Beyond Batten Disease Foundation? The foundation’s mission is to eradicate Batten disease by raising awareness of it and generating funding for research to cure and prevent this deadly illness. Batten disease is a rare, degenerative and fatal neurological disorder that children can inherit if both parents carry the genetic mutation for it. It strikes without warning, typically between ages 5 and 10. It first causes vision loss and seizures, then progressive impairment of cognitive and motor capacities, then death during the late teen years or early 20s. There currently are no treatments or a cure.
Why was the foundation started? Because Batten disease is rare (1.2 out of every 100,000 live births worldwide), there is very little federal funding for research on treatment and prevention. So we established the foundation to raise money for researchers to develop a cure and a test to screen for the genetic mutations that cause Batten disease and more than 600 other serious — and often fatal — conditions that kids can inherit. The hope with the test is to eliminate these devastating diseases in future generations. This strategy has proved successful on a small scale with Tay-Sachs disease.
What do you spend your money on? Ninety-three percent of the money we raise — a huge percentage by nonprofit sector standards — goes toward programs (versus operating costs) to accelerate the discovery of a treatment and the test that screens for genetic mutations. The foundation gave a $500,000 research grant to Texas Children’s Hospital and very generous friends of ours donated another $2 million. We’ve also invested $1.5 million with the National Center for Genome Resources to develop the carrier-screening test.
Talk about your Run to the Sun relay. The overnight relay run starts Saturday at 2:30 p.m. atop Austin’s scenic Mount Bonnell and finishes at sunrise May 15 at Enchanted Rock State Natural Area. That morning there will be a 6 to 8:30 a.m. breakfast celebration featuring live music by the Gospel Stars. About 30 teams, each with up to eight members, have registered to run the 95-mile course through the Texas Hill Country. Each team has pledged to raise a minimum of $5,000.
Is there still time to participate? Interest in Run to the Sun has been overwhelming. We’ve already exceeded our projected number of teams and runners. There’s still time to register a team, and you can make a donation or come out to cheer on the runners. Our biggest need at this point is for volunteers, so we encourage anyone who has time next weekend to contact us through the website (http://runtothesunrelay.com).
What do you see for your future? Every day is a blessing and we cherish each moment with our precious Christiane and our son, Garland. At the same time, we know that the years ahead will be challenging for her and our family. Nonetheless, we remain very hopeful as we raise money to accelerate research for developing a treatment for Batten disease and the screening test. We will find a treatment for Christiane and other affected children and ultimately eradicate Batten disease.
Run to the Sun
A year ago, Lance Thompson, our good friend and avid runner, came to us with an idea to organize a 100-mile overnight relay run to raise money for the foundation. Admittedly, I’m not a runner, and my first reaction was, “Who in the world is going to want to do that?!!” But as the idea evolved, and he shared his vision of a race whose course would meander under the starlit sky of the Texas countryside and culminate at a stunning destination at sunrise, I began to understand. Now, less than two weeks away, that vision will become a reality on May 14 when 30 teams of eight people each will compete by running a rugged Hill Country 96-mile course starting at Mount Bonnell and ending in the dawn light at Enchanted Rock. Members of each team will follow the route together in a van to support their runner and cheer him on as he steps onto the course alone to face his own unique challenge in the dark. Lance wanted the participants to experience first-hand the physical darkness and challenges that a child who is blinded by Batten Disease faces. It has been transformative to watch Lance’s ambitious dream become a reality and I am struck by how incredibly this race also mirrors our own life, and so perfectly mimics the mission of the Beyond Batten Disease Foundation. I love the way God inspires us only later to reveal His full intention.